title: Digestive tract symptoms in congenital langerhans cell histiocytosis: a fatal condition in an illness usually considered benign.
abstract: BACKGROUND
  Congenital Langerhans cell histiocytosis is usually limited to cutaneous lesions and has a good prognosis. In rare cases of gut involvement, mortality is high and early and aggressive treatment essential.
  
  
  METHODS
  We report a case of histiocytosis in a newborn with bowel involvement, and performed a literature review of 13 similar cases worldwide documented between 1973 and 2008.
  
  
  RESULTS
  Skin eruptions are usually the initial symptoms at birth. Bloody stools or protein-losing enteropathy are the first signs of bowel involvement that appear mostly in the first 4 weeks of life. Risk organs (hematopoietic system, liver, spleen) are often affected in the newborns with intestinal Langerhans cell histiocytosis. Prognosis is usually poor, with 78.5% mortality.
  
  
  CONCLUSIONS
  Even if histiocytosis in a neonate appears limited to autoinvoluting skin lesions, it is important to exclude all other organ involvement, including the bowel and stomach, as early treatment is vital.
authors.affiliation: *Department of Pediatrics, Division of Neonatology †Department of Pediatrics, Division of Oncology ‡Department of Dermatology, Hospital Universitari Son Espases, Palma de Mallorca, Spain.
authors.forename: Susanne
authors.initials: S
authors.lastname: Vetter-Laracy
authors.forename: Jose Antonio
authors.initials: JA
authors.lastname: Salinas
authors.forename: Ana
authors.initials: A
authors.lastname: Martin-Santiago
authors.forename: Mercedes
authors.initials: M
authors.lastname: Guibelalde
authors.forename: Pere-Ramon
authors.initials: PR
authors.lastname: Balliu
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D004066:Digestive System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D007231:Infant, Newborn; D011241:Prednisone / Q000627:therapeutic use*; D012074:Remission Induction; D014747:Vinblastine / Q000627:therapeutic use*
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D005938:Glucocorticoids; D014747:Vinblastine; D011241:Prednisone